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Acute-On-Chronic Liver Failure Review articles Acute-On-Chronic Liver Failure Verónica Pérez Guerra, MD1, Laura Ramírez Cardona, MD1, Oscar Mauricio Yepes Grajales, MD1, Juan David Vélez Rivera, MD2, Juan Ignacio Marín Zuluaga, MD3 1 Gastrohepatology Group of the School of Medicine at Abstract the Universidad de Antioquia in Medellín, Colombia 2 Internist and clinical hepatologist at IPS Universitaria Acute-on-chronic liver failure (ACLF) includes acute deterioration of liver functions in patients with either in Medellín, Colombia chronic de novo liver disease or already diagnosed liver disease. ACLF can be triggered by various hepatic 3 Internist - clinical hepatologist at the Hospital or extrahepatic precipitating factors. Among its clinical manifestations are renal dysfunction, hepatic encepha- Pablo Tobón Uribe and member of the Liver Transplant Group and Gastrohepatology Group lopathy, and multisystem organ failure. If not treated promptly translate the prognosis can be poor. Several at the Universidad de Antioquia, Professor at the scoring systems have been used to assess liver function and patient prognosis. Although multisystem organ Universidad Pontificia Bolivariana in Medellín, failure contraindicates liver transplantation, it remains the treatment of choice for this patients. Colombia ......................................... Keywords Received: 29-09-15 Acute-on-chronic liver failure, MARS, hepatorenal syndrome, hepatic encephalopathy, multisystem organ Accepted: 25-07-16 failure, immune paralysis, SOFA scores. INTRODUCTION a high risk of death. Consequently, treatment and mana- gement of ACLF must be different from those appropriate Acute on chronic liver failure (ACLF) is an entity that has for decompensated cirrhosis. increasingly gained recognition but that still generates The Asian Pacific Association for the Study of the Liver controversy because of the lack of a consensus definition (APASL) issued on of the first definitions of ACLF as a since it covers acute deterioration of liver function in condition that develops in the context of a patient who may patients with known chronic liver disease as well as newly or may not have been diagnosed with chronic liver disease diagnosed diseases. (1) The clinical concept of ACLF and who suffers an acute insult manifested by jaundice and seeks to differentiate this condition from decompensated coagulopathy and which can become complicated within liver cirrhosis as shown in Figure 1. The hepatocellular the first four weeks by ascites and/or encephalopathy. functioning of patients with liver cirrhosis slowly dete- (2) The European Association for the Study of the Liver riorates to a point at which decompensation occurs and (EASL) and the American Association for the Study of is manifested by complications associated with portal Liver Diseases (AASLD) define this entity as an acute dete- hypertension. At this point, the only treatment option rioration of a chronic underlying liver disease triggered by is liver transplantation. In contrast, patients with ACLF a precipitating event that leads to increased 3 month rates have more or less preserved liver functions but experience and multisystem organ failure. (3) a precipitating hepatic or extrahepatic event such as an Recently the European Association for the Study of the infection. Because of the exaggerated immune response, Liver Chronic Liver Failure (EASL CLIF) Consortium this quickly develops into multisystem organ failure with has defined acute on chronic liver failure based on the 260 © 2016 Asociaciones Colombianas de Gastroenterología, Endoscopia digestiva, Coloproctología y Hepatología Acute insult 100 Threshold for organ failure Liver function (%) Irreversible failure Chronic decompensated cirrhosis 0 Months Figure 1. The figure represents acute-on-chronic liver failure and differentiates it from chronic decompensated cirrhosis. The red line shows the progression of decompensated cirrhosis to organ failure at which point, due to the advanced stage of liver disease, opportunities to reverse liver disease are scarce, and the only treatment option is liver transplantation. The green line shows the development of ACLF in which sometimes good liver functioning deteriorates sharply due to a precipitating event which may lead to organ failure and a high risk of death. The patient may have advanced liver disease and appear to be stable but deteriorate sharply thanks to the precipitating event, and then progress to organ failure. This type of patient, in contrast those with advanced decompensated cirrhosis, has a potential for reversal and recovery to the state that existed prior to the precipitating event. Modified from Jalan R, Gines P, JC Olson, RP Mookerjee, Moreau R, Garcia-Tsao G, et al. Acute-on-chronic-liver-failure. J Hepatol. 2012; 57 (6): 1336-1348. CANONIC study. The study analyzed development of her mortality rate than patients who had had prior acute organ dysfunction according to the criteria of CLIF-SOFA decompensation. (6) (defined later in this text) in cirrhotic patients with acute decompensation which was defined as encephalopathy, DIAGNOSTIC CRITERIA ascites, gastrointestinal tract bleeding and bacterial infec- tion. (4). This study demonstrated that ACLF patients The lack of diagnostic criteria is currently one of the biggest exist and have prognoses different from those of patients problems with ACLF. In 2013, the CANONIC study set with decompensated cirrhosis. a goal of establishing diagnostic criteria for ACLF based Due to the absence of a consensus definition, it has been on analysis of patients with organ failure as defined by the difficult to estimate the incidence and prevalence of ACLF, CLIF-SOFA score (Table 1) and the at 28-day mortality Nevertheless, the CANONIC study, which had a sample of rate as shown in Table 2. The study shows that most patients 1,343 patients hospitalized for acute decompensated cirr- with ACLF were young alcoholics who had associated hosis in 29 specialized centers in 8 European countries, had bacterial infections and who had higher white blood cell made it possible to calculate a prevalence of 31% for ACLF. counts and levels of C-reactive protein (CRP) than those The study also found that the mortality rate among ACLF found in patients without ACLF. The CLIF-SOFA score, patients was 34% in the first 28 days while it was only 1.9% which was higher in patients with ACLF, and leukocyte for patients without ACLF. (4) counts were independent predictors of mortality in these Other studies have estimated that 40% of patients with patients. Accordingly, in addition to organ failure and high advanced cirrhosis over a period of 5 years could develop mortality rates, diagnosis of ACLF should also consider age ACLF. (5) This same study identified that patients who at presentation, the precipitator of the event precipitant, had no prior episodes of acute decompensation had a hig- and any systemic inflammation which may develop. (4) Acute-On-Chronic Liver Failure 261 Table 1. CLIF-SOFA score. Organ/system 0 1 2 3 4 Liver (bilirubin mg/dL) <1.2 ≥1.2 to <2.0 ≥2.0 to <6.0 ≥6.0 to <12 ≥12.0 Kidney (Creatinine, mg/dL) <1.2 ≥1.2 to <2.0 ≥2.0 to <3.5 ≥3.5 to <5.0 ≥5.0 Cerebral (hepatic encephalopathy (HE stage) No HE I II III IV Coagulation (INR) <1.1 ≥1.1 to <1.25 ≥1.25 to <1.5 ≥1.5 to <2.5 ≥2.5 or platelet count ≤20x109/L Circulation (MAP, mmHg) ≥70 <70 Dopamine ≤5 or Dopamine >5 or Dopamine >15 or Dobutamine or Terlipressin E ≤0.1 or NE ≤0.1 E >0.1 or NE >0.1 Lungs <400 >300 to ≤400 >200 to ≤300 >100 to ≤200 ≤100 PaO2/FiO2 o SpO2/FiO2 >512 >357 to ≤512 >214 to ≤357 >89 to ≤214 ≤89 The CLIF-SOFA score is a modification of the SOFA (Sequential Organ Failure Assessment) score developed for the CANONIC study because the SOFA score did not take into account specific characteristics of patients with cirrhosis. Like the SOFA score, the CLIF-SOFA score ranks organ failure from 0 to 4 in increasing order of organ/system disability. HE grades correspond to the West-Haven classification. Areas in bold indicate organ/system failure. HE: hepatic encephalopathy, INR: international normalized ratio, MAP: mean arterial pressure, E: epinephrine. NE:norepinephrine, PaO 2: arterial oxygen partial pressure, FiO 2: fraction of inspired oxygen, SpO2: pulse oximetry saturation. Modified from Reference 4. Table 2. Definition of ACLF EASL-CLIF CLIF/SOFA score definition of organ failure Liver failure defined by bilirubin of 12 mg/dL or higher. Renal failure defined by creatinine of 2 mg/dL or higher or by renal replacement therapy. Brain damage defined by West Haven encephalopathy the criteria Coagulopathy defined by INR of two platelets or less in 20,000 Circulatory dysfunction defined as the need for vasoactive or inotropic drugs Pulmonary dysfunction defined as PF ration of less than 200 Definition of ACLF No ACLF: 3 groups 1. Patients without organ failure. 2. Patients with failure of only one organ (liver failure, circulatory, respiratory or coagulopathy) whose creatinine level is under 1.5 mg/dL and who do not have encephalopathy. 3. Patients who only have encephalopathy and whose creatinine level is below 1.5 mg/dL. * The mortality rate is 4.7% at 28 days and 14% at 90 days. ACLF 1: 3 groups 1. Patients with renal failure alone 2. Patients with one organ failure but without renal failure who have creatinine levels below 1.5 mg/dL and who do not have encephalopathy 3. Patients with encephalopathy whose creatinine levels are between 1.5 and 1.9 mg/dL. * The mortality rate is 22.1% at 28 days and 40.7% at 90 days. ACLF 2: Patients with failure of two organs * The mortality rate is 32% at 28 days and 52.3% at 90 days ACLF 3: Patients with failure of three or more organs * The mortality rate is 76.7% at 28 days and 79.1% at 90 days Taken from Reference 9.
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